Introduction A sarcoma is a soft tissue, connective tissue or a bone cancerous malignant tumor. They can occur in either children or adults. For children under 20, 15% of cancer cases are sarcomas. 1 Rhabdomyosarcoma (RMS) is a type of sarcoma that is caused by a disruption in the pathway of primitive mesenchymal stem cells directed towards myogenesis. 2 Although RMS is a very rare form of cancer, it is the most common pediatric sarcoma primarily affecting children before the age of 5, yet is very rare in adults. 3 From the year 2006 to 2010 there has been 295 cases of children diagnosed with RMS in Canada, with 43 mortalities from the year 2008 to 2012. 4 RMS is 1.4 times more likely in males than females, and it is not different among different races of ethnicities. 5 There are 4 major types of RMS. Embryonal rhabdomyosarcoma (ERMS) is the most common type of RMS representing 70% of cases, and it usually affects children under 10 years of age. The other three types include alveolar rhabdomyosarcoma (ARMS), pleomorphic rhabdomyosarcoma (PRMS), and sclerosing/spindle cell Rhabdomyosarcoma, which comprise the remaining 30%. 5 We have summarized the distribution of different RMS subtypes in Figure 1. Patients with RMS can show symptoms including swelling, proptosis directly related to mass, and abdominal pain. Primary tumors can occur in different sites in the body such as head/neck (para-meningeal, orbital, head and neck), genitourinary (prostate, vaginal tract, bladder, and para-testicular), and extremity. But different types of RMS have their own common specific sites, for example the most common sites for ERMS tumors are the head and neck area of the patients. 6 Furthermore, different forms of RMS display different forms of onco- doi 10.15171/ijbsm.2017.01 Biologic and Clinical Aspects of Rhabdomyosarcoma Arya Emami 1 , Zahra Sepehri 2 , Joseph W. Gordon 3,4,5† , Saeid Ghavami 5,6†* 1 Department of Human Kinetics, Faculty of Biological Sciences, University of Guelph, Guelph, Canada 2 Zabol University of Medical Sciences, Zabol, Iran 3 Rady Faculty of Health Sciences, Max Rady College of Nursing, University of Manitoba, Winnipeg, MB, Canada 4 Children Hospital Research Institute of Manitoba, University of Manitoba, Canada 5 Department of Human Anatomy and Cell Science, Rady Faculty of Health Sciences, Max Rady College of Medicine, University of Manitoba, Winnipeg, MB, Canada 6 Biology of Breathing Theme, Children Hospital Research Institute of Manitoba, University of Manitoba, Canada Abstract Rhabdomyosarcoma (RMS) is a muscle-derived tumor and is the most common pediatric soft tissue sarcoma representing 5% of all childhood cancers. Statistically, soft tissue sarcomas account for approximately 10% of all cancers in children, of which more than half of these tumors are RMS. Thus, RMS is a major clinical problem in pediatric oncology. RMS is caused by a disruption in the pathway of primitive mesenchymal stem cells directed towards myogenesis. In most cases of patients diagnosed with RMS there is a genetic or chromosomal alteration involved. In past few years there have been discoveries of more therapeutic approaches that has improved the quality of life in RMS patients and has resulted in a better survival rate in this population from 25% to 60%. However, Additional researches and clinical trials are needed in order to minimize the devastating consequences of the pediatric cancer including RMS. In the current mini review we will briefly discuss current knowledge in RMS focusing on most common biological and clinical aspects of the disease. Keywords: Rhabdomyosarcoma, Childhood cancer, Cancer therapy. *Correspondence to Saeid Ghavami; Rady Faculty of Health Sciences, Max Rady College of Nursing, University of Manitoba, Winnipeg, MB, Canada. Tel: +1 204 272 3061; Email: saeid.ghavami@umanitoba.ca † These authors have equal senior authorship. Received January 23, 2017 Accepted February 15, 2017 Published online 5 March 2017 Int J Basic Sci Med. 2017;2(1):1-4 Mini-Review http://ijbsm.zbmu.ac.ir Copyright © 2017 The Author(s); Published by Zabol University of Medical Sciences. This is an open-access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Please cite this article as follows: Emami A, Sepehri Z, Joseph W. Gordon JW, Ghavami S. Biologic and clinical aspects of rhabdomyosarcoma. Int J Basic Sci Med. 2017;2(1):1- 4. doi:10.15171/ ijbms.2017.01.